Discussion:
Of note in this case report is the fact that crizanlizumab, originally approved by the FDA for the treatment of sickle cell-associated vaso-occlusive crisis based on the SUSTAIN trial[6], appears to have played an important role in the complete disappearance of priapism in our patient. This finding, although an isolated case, provides a new direction for the study and treatment of priapism in SCD patients and warrants further investigation using real-world data.
The infusion-related reaction (IRR) that occurred in our patient during the second crizanlizumab infusion, while concerning, is consistent with previous reports of rare but still significant adverse events during the initial phase of this treatment regimen. In our patient’s experience, the occurrence of IRR is not indicative of a recurrence of the episode and does not compromise the long-term benefit of treatment. This finding should encourage physicians to consider continuing crizanlizumab treatment even after a single IRR event, no doubt after careful risk-benefit assessment [7]. However, our case emphasises the urgent need for guidance on the management of such infusion-related reactions.
The overall results of crizanlizumab administration in our patient were positive, confirming its benefit in the management of vaso-occlusive crises in SCD and its potential benefit in stuttering priapism. Nevertheless, future studies with a larger cohort and longer follow-up periods are urgently needed to validate these results and to better understand the unseen facets of crizanlizumab therapy.